Polymorphous adenocarcinoma of the submandibular gland

Polymorphous adenocarcinoma (PA) of the salivary glands is a rare malignancy that predominantly affects the minor salivary glands of the palate. Major salivary gland involvement is rare (<5%). The submandibular gland is a highly unusual location for this tumour. Recently, the WHO has updated the classification of salivary gland tumours in which the PA subtype has been modified. We report a very uncommon case of a classical variant of PA involving the submandibular gland in a 49-year-old woman managed at our institute and discuss the most recent pathological criteria for diagnosis, management strategy and prognosis of PA.